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Biomedical subjects

P R Camfield

Publications and source records attributed to P R Camfield.

At least 19 recordsLinked to original sources
PubMed2004

Paroxysmal eyelid movements: a confusing feature of generalized photosensitive epilepsy.

BACKGROUND: Persistent, frequent, nonepileptic paroxysmal eyelid movements were observed in 19 children and adults with well-controlled generalized epilepsy. METHODS: Patients were identified from five epilepsy centers. RESULTS: Seventeen patients were female and two male. All had generalized photosensitive epilepsy requiring antiepileptic drugs (AEDs). In two children, paroxysmal eyelid movements began 2 to 4 years before their epilepsy was noted; in the remainder, it was noted when epilepsy was first diagnosed. Age at last follow-up was 8 to 38 years (average 21 years) with average follow-up of 9 years. All patients showed photosensitive generalized spike-wave discharges on EEG. Paroxysmal eyelid movements were a source of diagnostic confusion, but direct examination and video during EEG recording distinguished the attacks from absence seizures. In all cases, the epilepsy is completely or nearly completely controlled with AEDs, but the paroxysmal eyelid movements have not resolved with age. In 12 cases, there was a family history of the eyelid disorder without epilepsy. Videos of patients and an affected parent are available on the Neurology Web site. CONCLUSION: There is an association between paroxysmal eyelid movements and photosensitive generalized epilepsy, creating diagnostic confusion.

Adolescent↗
PubMed2002

Botulinum toxin A as a treatment for excessive drooling in children.

Drooling is problematic for some neurologically impaired children. Botulinum toxin A injection to salivary glands has effectively reduced drooling in adults but has only recently been used to treat children. This was a preliminary study to determine the efficacy and safety of botulinum toxin in children. Children identified as having severe daily drooling were enrolled. The preinjection assessment included measurement of the amount and frequency of drool. Each parotid gland was injected with 5 U of botulinum toxin A. Follow-up was for a minimum of 16 weeks. Nine children were enrolled, 4-17 years of age. All children had moderate or severe mental retardation. At week 4, all patients had a reduced drooling frequency and eight of nine patients had a reduction in the weight of saliva. Overall, five of nine parents (55%) deemed the treatment successful. This preliminary study demonstrates that botulinum toxin A is a relatively effective treatment for some children with significant drooling without serious side effects.

Adolescent↗
PubMed2002

Duchenne muscular dystrophy--parental perceptions.

Quality of life and availability of services are important for boys with Duchenne muscular dystrophy (DMD) and their families. Families attending our neuromuscular clinic completed a questionnaire on parental perception regarding the importance of services, health issues, and quality of life issues both "now" and "in the future." Eighty-nine percent of the families (31/35) completed questionnaires. Services and health issues related to prolonging ambulation were most important, especially for the parents of younger boys. Mental health issues such as social isolation, anger, and depression were very important, particularly for the families of older boys and were anticipated to be more important in the future. Pediatricians should be aware of both the immediate needs of families to meet the physical and emotional challenges of DMD and the increasing requirement to address the social needs of these patients and their families as the boys become older.

Adaptation, Psychological↗
PubMed2001

Population study of benign rolandic epilepsy: is treatment needed?

Forty-three of 79 children (54%) with benign rolandic epilepsy from a regional population were treated with antiepileptic drugs (AED); 36 (46%) were not. Physician advice was a major determinant of treatment choice. AED significantly reduced generalized seizures (p = 0.001) but did not reduce partial seizures. After 4 to 14 years and >900 seizures, all patients were in remission without medication or injury. Physicians may confidently offer a no-AED treatment strategy.

Anticonvulsants↗
PubMed2001

Irritability in Krabbe's disease: dramatic response to low-dose morphine.

Krabbe's disease is characterized by normal neonatal development with subsequent regression and profound, medically intractable irritability. Two female infants presented at 5 months of age with increasing irritability, abnormal motor control, and developmental regression. Investigations confirmed the diagnosis of Krabbe's disease. Maximal treatment of gastroesophageal reflux and nitrazepam 0.1 mg/kg by mouth three times daily were unsuccessful in controlling irritability. Morphine was initiated and titrated to 0.06 mg/kg by mouth every 6 hours in Patient 1 and 0.1 mg/kg by mouth every 8 hours in Patient 2, resulting in remarkably successful control of irritability. The diagnosis of Krabbe's disease is devastating for families and is compounded by the marked irritability. Management is difficult, but in these two infants, irritability was successfully controlled with low-dose morphine.

Analgesics, Opioid↗
PubMed2001

Treatment of febrile seizures: the influence of treatment efficacy and side-effect profile on value to parents.

OBJECTIVES: We examined parents' perception of the value of treatments designed to reduce the risk of febrile seizure recurrence. STUDY DESIGN: The families of 42 children with febrile seizures were recruited after pediatric or neuropediatric consultation. A mail questionnaire addressed the family's willingness to pay for a hypothetical treatment for febrile seizures with risk reductions for future febrile seizures of 25%, 50%, 75%, and 100%. The hypothetical clinical scenario was then modified to include the side- effect profiles of either daily phenobarbital or valproic acid, or intermittent diazepam prophylaxis. Covariates included the nature of the child's febrile seizure(s), parents' familiarity with febrile seizures, experiences at the time of febrile seizures or with medication side effects, education and income, and mastery and trait anxiety. RESULTS: Thirty-eight parents, representing 22 of 42 families, completed questionnaires. There was a dramatic inflection in parents' willingness to pay for 100% risk reduction as opposed to 75% or lower risk reductions. Introduction of side effects dramatically reduced the value attached to each level of treatment benefit. Nevertheless, a few parents (3/38) would pay "as much as it takes" to be rid of their child's recurrence risk. CONCLUSIONS: Given the range of value assigned to prophylactic medication for febrile seizures, management strategies for children with febrile seizures must be responsive to the needs and values of individual families.

Adult↗
PubMed2000

Children with febrile seizures do not consume excess health care resources.

BACKGROUND: Febrile seizures are benign but so terrifying for parents that they may subsequently view their affected children as "vulnerable". Children viewed as vulnerable may be brought to medical attention more frequently. We examined subsequent hospitalizations and physician visits during a 6- to 7 1/2-year period for a group of children who had participated in a case-control study of initial febrile seizures. METHODS: Individual data from a regional cohort of 75 children with a first febrile seizure and 150 febrile and 150 afebrile controls were linked to 2 comprehensive provincial health services databases-a hospital admissions/ separations database and a physician services database. RESULTS: Linkage was achieved for 98% of the study cohort, with heath care utilization data for 6 to 7 1/2 years available for 96%. Children with febrile seizures had nearly identical rates of subsequent hospitalization compared with age-matched controls (chi2 test, P = .88). An excess of day-surgery visits for primarily otolaryngologic procedures was seen for the febrile seizure patients 0 to 12 months after their initial febrile seizure (chi2 test, P < .001). During the next 6 to 7 1/2 years, the febrile seizure patients had nearly identical rates of physician visits (chi2 test, P = .15); however, they had more visits to otolaryngologists in the first 3 to 9 months after the febrile seizure (chi2 test, P < .001), but fewer visits to pediatricians during the next 1 to 4 years (chi2 test, P < .001). CONCLUSIONS: Children with febrile seizures have nearly identical rates of hospital and physician services utilization compared with controls. This supports the hypothesis that febrile seizures are benign, and that parents recover from their initial anxiety and do not consider their children vulnerable to additional illness in the years that follow.

Case-Control Studies↗
PubMed2000

Treatment of children with "ordinary" epilepsy.

Many children with epilepsy have a relatively benign clinical course with eventual remission of their seizures and no further need for medication. It is not easy to be sure who these children are at the time of diagnosis, but they do not have catastrophic epilepsy. Epilepsy is best defined as two unprovoked seizures. Not all of these children require treatment and treatment is motivated by fear of brain damage, injury, death, kindling of additional seizures, and social consequences. None of these fears provides an absolute indication for treatment. The decision to start medication should be considered on an individual basis. The choice of a first AED is arbitrary with most AEDs having equal efficacy. Follow-up schedules have not been well studied. However, there is fairly convincing evidence that routine blood and urine screening for toxicity is of no benefit, if the child is asymptomatic. Serum drug levels are of little clear benefit. Once the child has been seizure-free for 6 months to 12 months, it is reasonable to consider stopping medication. Only rarely does seizure control fail to return if there are recurrences without medication.

Anticonvulsants↗
PubMed1999

Double-blind methylphenidate trials: practical, useful, and highly endorsed by families.

OBJECTIVE: To evaluate a 3-week, randomized, double-blind, methylphenidate placebo-controlled trial (MPT) in routine practice for children with attention-deficit disorder. PATIENTS AND METHODS: School-aged children with attention-deficit/hyperactivity disorder (Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition criteria) who enrolled an "N of 1" trial at a pediatric tertiary care center were eligible. Families (n = 50) with a child eligible for the MPT were given 3 bottles of identical capsules. The capsules contained, in random order: placebo of the prescribed dose of methylphenidate (Ritalin) hydrochloride (0.3 mg/kg or 0.6 mg/kg). Families gave the child 1 capsule at 8 AM and 1 capsule at noon. The family, teacher, and physician were blinded for the order of medication. Conners questionnaires (Conners Parent Questionnaire and Conners Teacher Questionnaire) and written comments were completed by parents and teachers at baseline and at the end of each week. Once MPT results were known and following discussion with the physician, families decided whether to continue methylphenidate therapy. Families were interviewed by telephone 14 to 21 months after the MPT. RESULTS: Forty-three (86%) of the 50 eligible children (mean age, 129 months) were contacted. No family found the MPT difficult, but 6 trials were incomplete, usually because of side effects. All families used the MPT to decide if methylphenidate was the correct treatment choice for their child and 68% (34 of 50 families) used the results exclusively. The remaining 16 families believed the MPT was helpful. Overall, 31 (72%) of the 43 children had a good response to methylphenidate treatment--20 (47%) continued to use it for longer than 12 months and 8 (26%) for 2 to 12 months; 3 responders chose not to use it after the MPT. Nine of the 43 families chose not to use methylphenidate treatment; however, all indicated that participating in the MPT helped them to make that decision. In follow-up interviews, the same proportion of methylphenidate users and nonusers reported improvement in many areas of function including significantly less time spent doing homework. Users reported reduced aggression (P<.001) and fewer discipline problems (P<.01) compared with nonusers. CONCLUSIONS: An "N of 1" MPT was easily performed and permitted families to decide whether to use methylphenidate for long-term treatment of attention-deficit disorder or attention-deficit/hyperactivity disorder. Regardless of methylphenidate use or lack of use, the condition of all of these children was improved at follow-up.

Attention Deficit Disorder with Hyperactivity↗
PubMed1999

The cognitive and behavioural effects of clobazam and standard monotherapy are comparable. Canadian Study Group for Childhood Epilepsy.

PURPOSE: To compare the cognitive and behavioural effects of clobazam versus standard monotherapy in the treatment of childhood epilepsy. METHODS: A randomized, double-blind, prospective design was carried out at three Canadian pediatric epilepsy centres. This study was part of a larger multi-centre study on the efficacy of clobazam. Children with newly diagnosed epilepsy were assigned randomly to receive clobazam or carbamazepine. Children who had failed previous treatment with carbamazepine were assigned randomly to clobazam or phenytoin. Children who had failed on any other antiepileptic drug were assigned randomly to receive clobazam or carbamazepine. In a subset of patients neuropsychological assessments were carried out at 6 weeks and 12 months after initiation of medication. Intelligence, memory, attention, psychomotor speed, and impulsivity were assessed. RESULTS: There were no differences between the clobazam and standard monotherapy groups on any of the neuropsychological measures obtained at 6 weeks or 12 months. There was no evidence for a deterioration in performance for those children who remained on clobazam for the entire 12-month study period. CONCLUSION: The cognitive and behavioural effects of clobazam appear to be similar to those of standard monotherapy.

Adolescent↗
PubMed1999

Topiramate in intractable childhood onset epilepsy--a cautionary note.

OBJECTIVES: To study the effectiveness and safety of topiramate in clinical practice, for a group of patients with childhood onset epilepsy. METHODS: All patients treated with topiramate at the three study centers between November 1995 and December 31, 1997 were analyzed retrospectively, using a standardized study protocol. Data were gathered on demographic features, seizures response and medication related adverse events. RESULTS: Eighty-seven patients were treated with topiramate. Over 90% seizure reduction was achieved in 8 (9%) patients, 50%-90% in 21 (24%), < 50% in 54 (62%) patients. Four patients (5%) had a deterioration in seizure control. Adverse events required topiramate discontinuation in 36 (41%). Of these 27 (31%) complained of unacceptable cognitive dulling. The rate of dose escalation and final dose in mg/kg were similar in those who remained on topiramate and those who were intolerant because of cognitive side effects. CONCLUSIONS: Although topiramate resulted in > 50% seizure reduction in 29 (33%) of this group of patients with difficult epilepsy, its usefulness was limited by a high incidence of adverse effects. Adverse events prevented ongoing therapy for 36 (41%) and cognitive dulling resulted in topiramate discontinuation by 27 (31%) of the group.

Adolescent↗
PubMed1998

A multicenter study of the efficacy of the ketogenic diet.

OBJECTIVE: To determine the efficacy of the ketogenic diet in multiple centers. DESIGN: A prospective study of the change in frequency of seizures in 51 children with intractable seizures who were treated with the ketogenic diet. SETTING: Patients were enrolled from the clinical practices of 7 sites. The diet was initiated in-hospital and the patients were followed up for at least 6 months. PATIENTS: Fifty-one children, aged 1 to 8 years, with more than 10 seizures per week, whose electroencephalogram showed generalized epileptiform abnormalities or multifocal spikes, and who had failed results when taking at least 2 appropriate anti-epileptic drugs. INTERVENTION: The children were hospitalized, fasted, and a 4:1 ketogenic diet was initiated and maintained. MAIN OUTCOME MEASURES: Frequency of seizures was documented from parental calendars and efficacy was compared with prediet baseline after 3, 6, and 12 months. The children were categorized as free of seizures, greater than 90% reduction, 50% to 90% reduction, or lower than 50% reduction in frequency of seizures. RESULTS: Eighty-eight percent of all children initiating the diet remained on it at 3 months, 69% remained on it at 6 months, and 47% remained on it at 1 year. Three months after initiating the diet, frequency of seizures was decreased to greater than 50% in 54%. At 6 months, 28 (55%) of the 51 initiating the diet had at least a 50% decrease from baseline, and at 1 year, 40% of those starting the diet had a greater than 50% decrease in seizures. Five patients (10%) were free of seizures at 1 year. Age, sex, principal seizure type, and electroencephalogram were not statistically related to outcome. CONCLUSION: The ketogenic diet is effective in substantially decreasing difficult-to-control seizures and can successfully be administered in a wide variety of settings.

Child↗
PubMed1998

Localization of the giant axonal neuropathy gene to chromosome 16q24.

Giant axonal neuropathy (GAN) is a degenerative disorder of the peripheral nerves that is inherited as an autosomal recessive trait, presenting in early childhood and progressing to death, usually by late adolescence. Diagnosis is made by peripheral nerve biopsy, in which a striking pathological finding is seen--fibers distorted by giant axonal swellings filled with densely packed bundles of neurofilaments (the primary intermediate filament in neurons), with segregation of other axoplasmic organelles. In addition to disorganized neurofilaments in nerve, disorganization of other members of the intermediate filament family of proteins is seen in other tissues; this implies that the underlying defect is one of generalized intermediate filament organization, with neurofilaments predominantly affected. We have pursued a genomewide search for regions of homozygosity of descent in 5 consanguineous families. A 5.3-cM region of homozygosity, shared in all 5 families, was found on chromosome 16q24, and linkage was established to this locus with a LOD score of 4.18 at theta = 0.00 at the most tightly linked marker, D16S3098. Determination of this locus is the first step toward characterizing the gene responsible for a fundamental property of intermediate filament organization and may shed light on other disorders (such as amyotrophic lateral sclerosis) in which neurofilament pathology is prominent.

Axons↗
PubMed1998

Outcome of neonatal stroke in full-term infants without significant birth asphyxia.

UNLABELLED: Seven children with neonatal stroke were identified and six were followed for 13 months to 5 years (mean 28 months). Gestational age ranged between 39 and 42 weeks and none had hypoxic ischaemic encephalopathy. Focal clonic seizures were the presenting feature in six, and one presented with apnoea. The age of the first seizure ranged between 8 and 48 h (mean 26). The total number of seizures ranged between 3 and 10 (mean 5), and all seizures resolved by day 3 in all cases. CT scan showed an ischaemic infarct in six cases, and one child had a haemorrhagic infarct in the right anterior cerebral artery distribution. Phenobarbital was maintained for 3 weeks to 6 months (mean 11 weeks). None had recurrent seizures beyond the 3rd day of life and all were developing normally with no significant focal neurological deficits on follow up assessment. CONCLUSIONS: Full-term infants with neonatal stroke unrelated to significant birth asphyxia have a favourable neurological outcome. Seizures appear to be restricted to the first 3 days of life. We recommend short-term treatment with anticonvulsants.

Anticonvulsants↗
PubMed1998

Nova Scotia Niemann-Pick disease (type D): clinical study of 20 cases.

Patients with Niemann-Pick type D have been traced to a single Acadian ancestor in Nova Scotia. The objective of this study was to describe the clinical course. A cohort of children with Niemann-Pick type D was identified by chart review. Some children were seen and a telephone interview with the remaining parents was conducted. Twenty children with Niemann-Pick type D were identified. The female to male ratio was 2:1. Five children had severe neonatal jaundice. Early milestones were normal in the majority. Neurologic symptoms generally developed between 5 and 10 years of age with a mean age of 7.2 years at diagnosis. Seizures developed in all between 4.5 and 16 years of age (mean, 10.5 yr), and were followed by significant physical and mental deterioration. The age at death ranged between 11 and 22.5 years (mean, 14.8 yr). In 61%, bronchopneumonia was the cause of death. There is significant variability in the presentation and clinical course of Niemann-Pick type D.

Age of Onset↗
PubMed1998

Children with familial cryptogenic epilepsy have a favorable seizure prognosis.

We examined the prognosis for children with epilepsy not conforming to a genetic syndrome, who also had close relatives with epilepsy. Probands, with no identified cause for epilepsy except a first-degree relative with epilepsy, were identified from a population-based cohort of 504 children in Nova Scotia, with onset of seizures between 1977 and 1985. The primary outcome measure was seizure remission after an average of 15 years follow-up for probands (n = 27) and 26 years for their affected relatives (n = 32). Of probands, 92% were seizure free for 3 or more years at the end of follow-up, compared with 76% of relatives. When seizures began before age 12 years, 96% of probands and 94% of affected relatives were seizure free at the end of follow-up. There was little concordance for the details of the clinical course between probands and affected family members. This high level of remission was considerably better than for similar patients from the original Nova Scotian cohort (P < .02). We conclude that children with epilepsy not conforming to a well-defined genetic syndrome, but with an affected first-degree relative, have a remarkably good prognosis.

Adolescent↗